Epidemiological Features and Prognosis of Retinoblastoma in Fars southern Iran, 2010-2018

Background: Retinoblastoma (RB) is the most common intraocular malignancy in childhood. The aim of this study was to investigate the epidemiological features and survival rates of patients with RB in southwestern Iran. Materials and Methods: This retrospective study was conducted on patients with RB referred to the only referral center of southwestern Iran from 2010 to 2018. Demographic characteristics at first symptom presentation, the time interval between the symptom presenting and the treatment, educational level, socioeconomic status, type of ocular symptoms, extra-ocular involvement, types of treatments, outcomes and follow-up, the treatment interval until death and the survival rate of the patient, and pathology reports were recorded and analyzed. Results: This study included 46 new patients with RB (25 boys and 21 girls) including 65.2% unilateral, 26.1% bilateral, and 8.7% trilateral involvements. The mean age at first symptom presentation was 18.98±16.16 months. The mean delay time was 2.48 (Interquartile range: 5.16) for boys and 3.02 (Interquartile range: 5.37) for girls (P = 0.265); death rate was significantly different for boys and girls (12.0 % versus 430%; P = 0.039). E-nucleation was done in 95.3% of cases. In 29% of patients the tumor was well-differentiated, and about 64.5% of the cases were pathologically graded at pathological tumor stages 3 or 4. At the time of the study, 54.3% of the patients were alive.  The mean survival time was 44.0 months. Conclusion: Almost all cases were diagnosed in the advanced stages of the disease in Southwestern Iran. The disease is not preventable but early diagnosis improves the prognosis so we recommend that an eye examination at birth and designing and implementing prevention programs through parenting and child care personnel be performed in order to pay attention to early symptoms of the condition and in the absence of symptoms, screening should be done every six months.